By Carol A. Kemper, MD, FACP

Dr. Kemper reports no financial relationships relevant to this field of study.

A Deadly Funeral

SOURCE: ProMED-mail post; Contaminated beer, fatal – Mozambique(03): (TETE) Burkholderia gladioli pathovar cocovenenans toxin. Nov. 9, 2015;

We’re so lucky in this country our water is clean, the food supply is generally reliable, and the beer won’t kill you. That was not the case in Mozambique last month. A home-made beer drunk by hundreds of people attending a funeral in Chitima, Mozambique, affected 232 people, causing 75 deaths. Many went to the hospital with diarrhea and severe muscle cramps, while some were simply found later that day dead in their homes. The woman who brewed the beer and her family were among the dead.

The culprit was a home-made beer, made from contaminated cornmeal flour. At first, it was thought the beer had been intentionally poisoned by crocodile bile. However, tests conducted in the United States identified Flavitoxin A, which is produced by lethal strains of Burkholderia gladioli. B. gladioli is a plant pathogen that rarely causes opportunistic infection in humans (e.g., cystic fibrosis patients), but some strains produce a potentially lethal toxin called bongkrekic acid, which, by spectrographic analysis, has been shown to be identical to Flavitoxin A. While the nomenclature for these organisms has been shifting around for years, microbiologists like to distinguish between non-toxin and toxin-producing strains, the latter of which have been renamed B. gladioli pathovar cocovenenans.

Bongkrekic acid took its name from the Indonesian practice of fermenting plant material, such as soybeans and coconut. Tempe bongkrek is made by fermenting coconut cake with Rhizopus oligosporus, which is naturally found in coconut. If it’s not adequately fermented, bacterial overgrowth can occur — similar to beer. A friend used to say that brewing beer was much harder than making wine, because of the risk of bacterial contamination. While we generally think of beer as produced from fermented barley (flavored with yeast and hops), people in developing countries make use of other starches, such as rice, corn, millet, sorghum, and cassava. Heat processing does not destroy the toxin. Food-related outbreaks of Flavitoxin A poisoning were reported in 2007 from fermented soybean cakes in Indonesia, and contaminated corn flour sacks in rural China.

Increase in Congenital Syphilis

SOURCE: ProMED-mail post Syphilis – USA: Pregnant women, congenital, rising incidence. Nov. 14, 2015.

Syphilis is popping up everywhere these days. I’ve never seen so many cases. ProMED-mail has been doing a nice job of summarizing the rising incidence of syphilis in a number of states, including California, Texas, Florida, New York, Ohio, and Indiana even in rural areas. What (re)emerged in the gay community, largely affecting men who have sex with men (MSM), has predictably begun to move into the heterosexual community. While most of my recent patients with syphilis are MSM, a recent patient was a 19-year-old woman (astounded to learn she had syphilis). I include syphilis screening on anyone being screened for STDs, as well as those with an unexplained sore throat, tonsillitis, cervical or inguinal lymphadenopathy, or any kind of unexplained rash regardless of their sexual history.

The national congenital syphilis (CS) rate reached an all-time low in 2010-2012, with 8.4 cases per 100,000 live births. Since then, the national rate has increased 38%, closely tracking with the national increase in syphilis cases in women. A few years ago, there were at most one or two annual cases of CS in the San Francisco Bay Area. In 2012, CS cases in California jumped to 35, and, in 2014, rose to 99 cases. So many cases have occurred in Fresno, CA, that the county public health officer has required all pregnant women to be screened three times during their pregnancy.

Many of these cases are occurring in immigrant and poor women, with delays in seeking prenatal care. While all pregnant women in California are eligible for MediCal, many don’t know how to access the care, and prenatal care and STD screening are often delayed, after permanent fetal damage has already occurred. Although syphilis in adults is readily treatable with penicillin, congenital syphilis is a severe deformative disease, with a high frequency of blindness and deafness; approximately 40% of affected infants die. Early detection in pregnancy is essential.

Clinicians are being urged to screen for syphilis with the first prenatal visit. In addition to weekly reviews of syphilis cases, and more aggressive contact tracing, public health authorities are being urged to prioritize female contacts of reproductive age. (Although this comes after many local health departments and STD clinics were gutted in 2008-2012; even our own county STD clinic and lab was closed for a few years. Does anyone see a correlation?)

I would recommend including RPR screening with any STD screening. Maybe we should return to the days of mandatory syphilis screening when applying for a marriage license, and RPRs should be included with hepatitis and HIV as part of “universal” screening.

That Is Not Strep Throat!

SOURCE: Smith JRM, et al. Tonsillar syphilis: An unusual site of infection detected by Treponemal pallidum PCR. J Clin Microbiol 2015;53:3089-3081.

Syphilis has been called the “Great Imitator” for a reason. Pick a symptom or a physical finding, and syphilis could be the cause. While older physicians (yikes, me) have had their share of exposure to this fascinating infection, many younger physicians have never seen a case.

These authors describe a young man with a steady male partner who had 2 weeks of sore throat and otalgia. He denied any outside sexual contact. His exam was remarkable for a large, shiny red tonsil and tender tonsillar adenopathy. Screening for group A strep was negative. He returned a week later, now anxious that maybe he had forgotten about an episode of oral sex with a stranger at a party several weeks earlier. His throat was still red, the tonsil now covered by a thin white exudate, and his adenopathy was still tender. Throat and urine NAAT studies for GC/chlamydia were performed, along with blood tests for an RPR, and a swab for T. pallidum DNA was obtained and sent to the National Microbiology Laboratory in Winnepeg, Canada. The RPR was positive at 1:16, and the PCR test (using three different gene targets) was positive. Sequencing revealed the A2058G mutation associated with macrolide resistance. Remarkably, by the time the studies came back and he was seen back in clinic for treatment, he was asymptomatic and his exam was unremarkable.

Put syphilis on your list for causes of exudative tonsillitis (I would have also included HSV culture). With the reported increase in oral sex, apparently favored by teens and young adults, be prepared to spot those cases of oropharyngeal and tonsillar syphilis. Primary syphilis may present in the oropharynx, with tonsillar erythema and exudative tonsillitis and often boggy, tender neck nodes. Fever may or may not be present. Children with syphilis (usually from kissing or pre-masticated food) also frequently present with oral findings and bulky tender nodes.

Another lesson here: Never believe a patient’s sexual history (at least not entirely); people “forget” their risk factors. If it’s appropriate to perform an STD test, don’t be dissuaded by a patient’s apparent lack of risk factors. And it’s reasonable to do RPR screening of high-risk patients on a regular basis.

Increase in Ocular Syphilis

SOURCEMMWR. Notes from the field: A cluster of ocular syphilis cases — Seattle, Washington, and San Francisco, California, 2014-2015. Oct. 16, 2015;64:1150-1151.

In 2014-2015, ocular syphilis was reported in four people from King County, WA, and in eight people from San Francisco County, CA. The first four cases, all of which occurred during a two-month period in late 2014, prompted a Clinical Advisory to medical providers and West Coast health departments. The median age of these four cases was 39 years (range, 29–52 years), three were HIV-positive, and their RPRs ranged from 1:252–1:4096. Among the three HIV-positive cases, the median CD4 count was 111 cells/mm3, and the median HIV RNA viral load was 34,740 copies/mL. All four patients presented with blurry vision, bright flashing lights and visual loss, and all four were diagnosed with uveitis. Three of the patients were believed to have early latent disease, and one had late latent disease, at least based on their histories.

Cerebrospinal fluid analysis was performed in three patients, and CSF FTA was positive in two. Despite treatment, two patients became legally blind during the next 5 months, and one patient had a permanent blind spot. The fourth patient was lost to follow-up.

As the result of the advisory, San Francisco County identified eight cases of ocular syphilis, occurring from December 2014 through March 2015. The median age of these cases was 52 years (35-58 years), and seven of the cases were HIV-positive, including one female sex worker. The median CD4 count was 291 cells/mm3, and the median HIV-RNA was 84,500 copies/mL. Ocular presentations included uveitis, optic neuritis, and one case of retinal detachment. Four of the cases had CSF analysis, three of which were positive for neurosyphilis. Following treatment, one patient had permanent visual loss in one eye.

RPRs ranged from 1:256–1:8192; two of the patients initially had negative RPRs because of the prozone affect.

Ocular syphilis — another great imitator — is an important cause of uveitis and optic neuritis, and is frequently associated with neurosyphilis. While it generally occurs during early syphilis infection, it is frequently associated with invasion of the central nervous system. These patients deserve cerebrospinal fluid analysis, especially if they are HIV-positive. Ocular syphilis should be managed just like neurosyphilis, regardless of the CSF results.